What is the Shenlein-Genoch Disease?
Hemorrhagic vasculitis is one of the most common hemorrhagic diseases, based on multiple microthrombuscovulitis, which affects the vessels of the skin and internal organs. The disease is often found in children and among children younger than 14 years old is observed with a frequency of 23-25 per 10 000.
Causes of Shenlein-Genoch Disease
At present, hemorrhagic vasculitis is proved to be an immunocomplex disease in which microvessels undergo aseptic inflammation with more or less deep damage to the walls, thrombosis and the formation of circulating immune complexes (CIC).
Pathogenesis during the Disease of Shenlein-Genoch
The reason for the development of this pathology is the formation in the bloodstream of circulating immune complexes. These substances are deposited on the inner surface of blood vessels, thereby causing their damage.
Damage to the blood vessels by circulating immune complexes in hemorrhagic vasculitis is not specific. It can be triggered by various factors: viral and bacterial (streptococcal) infections, vaccinations, drug allergies, food, parasitic invasions, cold. The first manifestations of vasculitis and its exacerbations are often accompanied by urticaria and other allergic rashes.
Symptoms of Illness Shenlein-Genoch
Classification
This reference book provides a classification of hemorrhagic vasculitis by G. A. Lyskina (2000).
- Form (evolution) of the disease:
– initial period;
– improvement;
– aggravation. - Clinical forms:
– simple;
– mixed. - Clinical syndromes:
– cutaneous;
– articular;
– abdominal;
– renal. - Severity
Easy:
– general condition is satisfactory;
– scanty rash;
– possible arthralgia.
Moderately heavy:
– general condition – moderate;
– profuse rash;
– arthralgia, arthritis;
– recurrent abdominal pain;
– microhematuria;
– small proteinuria (traces of protein in the urine).
Heavy:
– general condition – severe;
– plentiful discharge rashes with elements of necrosis;
– chronic angioedema;
– persistent abdominal pain;
– gastrointestinal bleeding;
– gross hematuria;
– nephrotic syndrome;
– acute renal failure.
The nature of the flow:
– acute (up to 2 months);
– prolonged (up to 6 months);
– chronic.
Clinic
In hemorrhagic vasculitis, the vessels of any area, including the lungs, brain, and its membranes can be affected.
Skin syndrome occurs most frequently. When it is symmetrically affected limbs, buttocks, at least – the torso. There is a papular-hemorrhagic rash, sometimes with blisters. Rash of the same type, first have a distinct inflammatory basis, in severe cases, complicated by central necrosis and covered with crusts, leave pigmentation for a long time. When pressed, the elements of the rash do not disappear.
Articular syndrome often occurs together with cutaneous or several hours or days after it in the form of pains of varying intensity in large joints (knee, elbow, hip). After a few days, the pain goes away, but with a new wave of rashes it may occur again. In some cases, joint damage is persistent and stubborn, resembles rheumatoid polyarthritis.
Abdominal syndrome is more often observed in children (in 54-72% of patients), approximately in 1/3 it prevails in the clinical picture, in some cases precedes skin changes, which makes diagnosis very difficult. The main symptom is severe pain in the abdomen, persistent or cramping, sometimes so intense that patients do not find a place in bed and cry for many hours. The pain is caused by hemorrhages in the intestinal wall. These hemorrhages can be combined with blood soaking the intestinal wall and mucous membrane, bleeding from it and from areas of necrosis, bloody vomiting, melena (blood admixture in the feces) or fresh blood in the feces, as well as false urges with frequent stools or, conversely, with his delayed. From the very beginning, fever, more or less pronounced leukocytosis (an increase in the number of leukocytes in the blood) are determined. In case of heavy bleeding, collapse (fainting) and acute post-hemorrhagic anemia develop. In some cases, frequent vomiting leads to a large loss of fluids and chlorides. In a coagulogram, hypertrombocytosis and hypercoagulation are determined.
In a significant proportion of patients, abdominal syndrome is short and goes away on its own in 2-3 days. Periods of severe pain can alternate with painless intervals that last about 1–3 hours. This helps to distinguish abdominal syndrome from acute surgical diseases of the abdominal organs. Such differentiation is especially difficult in patients without skin-articular manifestations and with symptoms of peritoneal irritation. More often the abdominal syndrome imitates acute intestinal obstruction (invagination), appendicitis, torsion and ovarian cysts, perforation of an ulcer of the intestine.
Comparative diagnostics can cause certain difficulties for the doctor – this is due to the fact that the hemorrhagic vasculitis itself can cause all of the listed surgical diseases of the abdominal organs. For example, many cases of invagination (introduction of one part of the intestine into another) and obstruction of the intestine due to compression or closure of its lumen with hematoma (especially in children younger than 2 years old), necrosis of the intestine and its perforation (formation of a through defect), acute appendicitis and other complications requiring surgical intervention. Difficulties of differential diagnosis in a similar situation lead to the fact that a part of patients with hemorrhagic vasculitis undergo unreasonable surgical interventions.
In adult patients, abdominal syndrome is less common and in most cases does not serve as a basis for diagnostic laparotomy, rarely complicated by intestinal obstruction and peritonitis (inflammation of the peritoneum). In old age, there is sometimes an abdominal version of the disease with uncertain and not always severe abdominal pains and persistent intestinal bleeding, the source of which cannot be determined. In search of a malignant neoplasm, hidden ulcer of the intestine or a bleeding polyp in such cases, they often go for a trial laparotomy and a wide examination of the abdominal organs. In old age with hemorrhagic vasculitis, such an operation, which does not give any tangible results, ends, as a rule, with atony (complete lack of tone) of the intestine and dynamic intestinal obstruction, a sharp increase in general intoxication, the addition of cardiovascular insufficiency and death of the patient. Meanwhile, the correct recognition in such cases of Shenlein-Genoch disease or even a trial course of treatment of this disease in diagnostically unclear cases allows you to quickly stop all symptoms and avoid unsaved and dangerous surgical intervention.
Kidney syndrome is found in 1/8-1/2 of patients and often develops as acute or chronic glomerulonephritis – with micro- or gross hematuria (blood in the urine), proteinuria (from 0.33 to 30% protein in the urine). Arterial hypertension in this form of pathology of the kidneys is rare. Possible nephrotic syndrome. Kidney damage often does not occur immediately, but within 1-4 weeks after the onset of the disease. Signs of jade can persist only a few weeks or months, but there is also a protracted or chronic course of the disease, which dramatically worsens the prognosis. In some patients, kidney damage progresses rapidly with outcome in uremia in the first 2 years of the disease. In general, kidney damage is a potentially dangerous manifestation of hemorrhagic vasculitis, and therefore the attending physician should closely monitor the composition of urine and kidney function throughout the course of the disease.
Much less frequently detected vascular lesion of the lungs, sometimes leading to the development of fatal pulmonary hemorrhage. Also, in rare cases, the cerebral form of the disease develops, with headaches, meningeal symptoms (hemorrhages in the sheaths of the brain), epileptiform seizures (resembling seizures in epilepsy).
Often there is an increase in temperature (initially up to 38-39 °C, then subfebrile, i.e. below 38 °C), a small and unstable initial leukocytosis, an increase in ESR, an increase in serum globulin levels, hyperfibrinogenemia (increased plasma fibrinogen) . Anemia develops due to blood loss.
Diagnosing Illness of Shenlein-Genoch
The diagnosis of hemorrhagic vasculitis is made on the basis of clinical data, and it does not require additional studies to confirm. In the analysis of peripheral blood, leukocytosis of varying severity, increased ESR acceleration, neutrophilia (an increase in the number of neutrophilic leukocytes), eosinophilia (an increase in the number of eosinophils), and thrombocytosis (an increase in the number of platelets) are detected. Given the frequent damage to the kidneys, all patients need to systematically do urine tests. In the presence of changes in the urine produce research to assess the functional state of the kidneys. Due to the fact that 1/3 of patients may have DIC, it is advisable to regularly count the number of platelets, and during the height of the disease to study the patient’s hemostasis (clotting time of venous blood, resistance to heparin, the level of fibrinogen and fibrin in the blood).
Great difficulties are caused by timely diagnosis of complications of the abdominal appendicitis syndrome, invagination, intestinal perforation and peritonitis. Such children need joint observation by a pediatrician and a pediatric surgeon over time.
Treatment of Disease Shenlein-Genoch
Hospitalization and bed rest for at least 3 weeks is a prerequisite for therapy, then it is gradually expanded, as purpura exacerbations are possible, which are explained as orthostatic purpura.
It is necessary to avoid cooling and additional allergization of patients with food and drugs in every way. Exclude cocoa, coffee, chocolate, citrus fruits, fresh berries (strawberries, strawberries) and dishes from them, as well as individually intolerable types of food.
The use of antibiotics, sulfonamides and other allergenic drugs (including all vitamins) that can support hemorrhagic vasculitis or contribute to its aggravation should be avoided. Low-allergy antibiotics (ceporin, rifampicin) are prescribed only for background or associated acute infectious diseases (for example, croupous pneumonia). Articular syndrome, an increase in body temperature, leukocytosis and increased ESR are not an indication for prescribing antibiotics and other antibacterial drugs, since they are characterized by immune aseptic inflammation.
All patients with hemorrhagic vasculitis are recommended to administer enterosorbents, such as activated carbon, cholestyramine, or polyphepan, orally. In addition, gastric drops, anti-allergic drugs (antihistamines), calcium pantothenate, rutin, average doses of ascorbic acid are prescribed, and phytotherapy is also used. With all this, the effectiveness of the above drugs in the treatment of this pathology remains highly questionable.
Patients have abdominal pains that do not go away with the reception of gastric drops, resort to the use of drugs with an anesthetic effect, such as no-shpa, baralgin.
The use of disaggregants, such as chimes, pentoxifylline (trental), is considered justified. The duration of treatment is 3 months. In case of moderate hemorrhagic vasculitis, it is recommended to use 2 disaggregants, and in case of chronic course of the pathology under consideration, add plavinil (delagil) to therapy. The duration of such therapy may last up to 1 year. It is also recommended the appointment of drugs with a membrane-stabilizing effect (vitamins A, E, dimefosfon).
High activity of the process with pronounced abdominal, skin and joint syndrome is an indication for prescribing a combination of the following drugs: prednisone and heparin. Isolated prescription of prednisolone is dangerous, since it contributes to the increase in blood clotting, and there is always a tendency to develop DIC in this disease (even if there are no clear signs of its presence). Prednisolone is usually prescribed in a dose of 1 mg/kg, and heparin – 200-300 U/kg per day, divided into 4-6 injections, under the skin of the abdomen. If, against the background of heparin therapy, the clotting time of venous blood continues to be shortened (less than 8 minutes), then the dose can be increased by 1.5 times. Heparin should not be administered 2 or 3 times a day, as it provokes the development of intravascular blood clots. Canceling heparin should be gradual, but at the cost of reducing the dose, not reducing the number of injections. Sometimes with a stormy clinical picture, one has to resort to infusion therapy, and in this case, it is possible to achieve the optimal administration of heparin — intravenously, with a steady flow of it into the body throughout the day.
In severe cases, in addition to heparin therapy and glucocorticoids, 5-8 plasmapheresis sessions are prescribed. The first three plasmapheresis sessions are carried out daily, the next – 1 time in 3 days. Fresh-frozen plasma, albumin and glucose solutions are used as replacement drugs.
Perhaps a combination of pulse therapy with prednisone (15-20 mg/kg/day for 3 days) and plasma exchange.
In patients with subacute nephritis or with the rapid course of glomerulonephritis, they resort to the combined prescription of immunosuppressants (azathioprine or cyclophosphamide) with glucocorticoids and heparin, antiplatelet agents (curantil). Cytostatics should not be prescribed only in connection with a protracted or undulating course of the disease. It is recommended that such patients be examined for the presence of worms, foci of infection, that is, to look for the cause.
With the right treatment, abdominal syndrome is usually resolved most quickly, the intensity of which often decreases already a few hours after the intravenous administration of heparin. The simplest (skin-articular) variants of vasculitis are most persistent. The periodic appearance of a small number of elements of rash on the legs and feet without other symptoms in general is often not amenable to therapy. Sometimes they are treated with local applications. These rashes are safe and after a while pass spontaneously.
There is evidence of repeated occurrence of exacerbation of hemorrhagic vasculitis due to psycho-emotional stress, hysterical background, stressful situations. The patient is provided with psychological peace, if necessary, recommended sedatives and tranquilizers, which increases the effectiveness of complex therapy.
Forecast
It is estimated that 60% of patients with hemorrhagic vasculitis recover within a month, and 95% within a year.
Chronic nephritis develops in 1-2% of patients suffering from this pathology. Mortality in hemorrhagic vasculitis is about 3% and even less due to forms with organ complications and cases of chronic nephritis.
Dispensary observation
If kidney damage is absent, children are in the dispensary at the district pediatrician for 5 years. Every six months a child is shown to the dentist, otorhinolaryngologist for the timely diagnosis and treatment of the most common foci of infection. Also regularly examine the feces for helminth eggs. At least once a quarter and after each transferred ARI, urine tests are performed. Medical exemption from vaccinations is given for 2 years. Planned therapy is not indicated.
Prevention of Illness of Shenlein-Genoch
In the prevention of exacerbations of the disease, an important role is played by the prevention of exacerbations of chronic infection, the refusal to take antibiotics and other drugs without valid indications (taking tetracyclines, levomycetin is particularly undesirable), excluding contact with allergens. Vaccinations and samples with bacterial antigens (for example, tuberculin) are contraindicated in patients, since they often cause severe relapses of the disease. Relapses can also provoke cooling, physical exertion, eating disorders, and alcohol.